Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ Syndrome consisting of alopecia or hypotrichosis, oligophrenia, microcephaly, seizures, and mental retardation. + Synonyms Download Section PDF Listen ++ Alopecia-Epilepsy-Oligophrenia Syndrome; Alopecia-Mental Retardation-Epilepsy-Microcephaly Syndrome; Epilepsy-Oligophrenia Syndrome of Moynahan. + Classification Download Section PDF Listen ++ Three types of this disorder are suggested: ++ Type I: Total alopecia and mental retardation, the main features, in association with microcephaly ++ Type II: Subtotal alopecia and mental retardation with or without epilepsy ++ Type III: Subtotal alopecia and psychomotor retardation with microcephaly and epilepsy + History Download Section PDF Listen ++ First described in 1962 by E.J. Moynahan, an British dermatologist practicing in London. + Incidence Download Section PDF Listen ++ The incidence remains unknown. + Genetic Inheritance Download Section PDF Listen ++ Familial with parental consanguinity; autosomal recessive trait. + Pathophysiology Download Section PDF Listen ++ Unknown. + Diagnosis Download Section PDF Listen ++ Microcephaly, alopecia or hypertrichosis, and epilepsy (grand mal seizures). + Clinical Aspects Download Section PDF Listen ++ Sparse or absent scalp hair, microcephaly, seizures, short stature; hypogonadism with late puberty. Poor musculature. Sometimes sensorineural deafness. + Precautions before Anesthesia Download Section PDF Listen ++ No literature on anesthesia in this condition. However, full neurologic history and examination, epileptic history, especially of control and medications, and associated anomalies resulting from microcephaly must be reviewed. + Anesthetic Considerations Download Section PDF Listen ++ Epileptic control is essential; usual medications should be maintained until the morning of surgery wherever possible and appropriate nontriggering anesthetic agents used. Psychomotor retardation with poor speech skills may lead to a uncooperative patient. + Pharmacological Implications Download Section PDF Listen ++ Note potential for interaction with antiepileptic drugs. Chronic phenytoin administration increases nondepolarizing neuromuscular blocker requirements, produces gingival hyperplasia and bleeding, and may cause hepatic dysfunction. Ketamine, enflurane, and methohexital are relatively contraindicated. High concentration (greater than 5-6%) of sevoflurane at induction might trigger epilepsy. + References Download Section PDF Listen ++Baraitser M, Carter CO, Brett EM: A new alopecia/mental retardation syndrome. J Med Genet 20:64, 1983. [PubMed: 6842537] ++Moynahan EJ: Familial congenital alopecia, epilepsy, mental retardation with unusual electroencephalogram. Proc R Soc Med 55:411, 1962. [PubMed: 14476762] ++Perniola T, Krajewska G, Carnevale F, et al: Congenital alopecia, psychomotor retardation, convulsions in two sibs of a consanguineous marriage. J Inherit Metab Dis 3:49, 1980. [PubMed: 6777601]